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Journal of Neuro-Oncology

, Volume 145, Issue 3, pp 519–529 | Cite as

Evaluating pediatric spinal low-grade gliomas: a 30-year retrospective analysis

  • Steven S. CareyEmail author
  • Zsila Sadighi
  • Shengjie Wu
  • Jason Chiang
  • Giles W. Robinson
  • Yahya Ghazwani
  • Anthony P. Y. Liu
  • Sahaja Acharya
  • Thomas E. Merchant
  • Frederick A. Boop
  • Amar Gajjar
  • Ibrahim Qaddoumi
Clinical Study

Abstract

Purpose

Most pediatric spinal tumors are low-grade gliomas (LGGs). Characterization of these tumors has been difficult given their heterogeneity and rare incidence. The objective was to characterize such tumors diagnosed at our institution.

Methods

Spinal tumors diagnosed in our pediatric patients between 1984 and 2014 were reviewed retrospectively. Demographics, presentation, pathology, imaging, management, and sequelae were examined.

Results

Forty patients had spinal LGG tumors, 24 (62%) of which were pilocytic astrocytomas. The most common initial presentations were pain (n = 15), partial extremity paralysis (n = 13), and ataxia (n = 11), with the diagnosis frequently delayed by months (median = 5.9 months, range 4 days–6.2 years). Twenty-nine patients had some tumor resection, and 8 required adjuvant therapy with chemotherapy (n = 4) or radiation (n = 4) post-resection. Ten other patients received only biopsy for histologic diagnosis, who were treated with chemotherapy (n = 4) or radiation (n = 5) post biopsy. Tumor progression was noted in 16 patients (2 after gross-total resection; 10, partial resection; and 4, biopsy). During the evaluation period, 3 patients died secondary to tumor progression. BRAF status could have shortened progression-free survival: patients with BRAFV600E mutations (n = 3) all experienced progression within 10 months. Long-term sequelae of the disease/treatment were mostly residual neurologic deficits (paresthesia, paralysis), chemotherapy-induced hearing loss, and scoliosis.

Conclusions

Spinal LGG is a rare entity with significant long-term effects. Although surgery is the most common initial treatment option, more in-depth analysis of molecular biomarkers may improve stratification and prognostication.

Keywords

Low grade glioma Pediatric Spinal Treatment Biomarkers BRAF 

Notes

Acknowledgements

We would like to thank Cherise Guess, PhD, ELS, for scientific editing of the final manuscript, and Laura Patterson, AuD, for help with audiogram interpretation.

Funding

This work was supported, in part, by Cancer Center Support Grant CA21765 from the National Cancer Institute and the American Lebanese Syrian Associated Charities (ALSAC).

Compliance with ethical standards

Conflict of interest

All authors report no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Our institutional review board reviewed and approved this retrospective chart review.

Supplementary material

11060_2019_3319_MOESM1_ESM.tif (313 kb)
Supplementary material (TIF 314 kb)

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Steven S. Carey
    • 1
    Email author
  • Zsila Sadighi
    • 2
  • Shengjie Wu
    • 3
  • Jason Chiang
    • 4
  • Giles W. Robinson
    • 1
  • Yahya Ghazwani
    • 1
  • Anthony P. Y. Liu
    • 1
  • Sahaja Acharya
    • 5
  • Thomas E. Merchant
    • 5
  • Frederick A. Boop
    • 6
    • 7
    • 8
    • 9
  • Amar Gajjar
    • 1
  • Ibrahim Qaddoumi
    • 1
  1. 1.Department of OncologySt. Jude Children’s Research HospitalMemphisUSA
  2. 2.Department of NeurologySt. Jude Children’s Research HospitalMemphisUSA
  3. 3.Department of BiostatisticsSt. Jude Children’s Research HospitalMemphisUSA
  4. 4.Department of PathologySt. Jude Children’s Research HospitalMemphisUSA
  5. 5.Department of Radiation OncologySt. Jude Children’s Research HospitalMemphisUSA
  6. 6.Department of SurgerySt. Jude Children’s Research HospitalMemphisUSA
  7. 7.Department of NeurosurgeryUniversity of Tennessee Health Science CenterMemphisUSA
  8. 8.Le Bonheur Neuroscience InstituteLe Bonheur Children’s HospitalMemphisUSA
  9. 9.Semmes Murphey ClinicMemphisUSA

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